Malignant proliferating trichilemmal cyst of the scalp: histological aspects and nosology.

BACKGROUND: Malignant proliferating trichilemmal cyst is a rare tumour usually located on the scalp of elderly women. About 40 cases of malignant proliferating trichilemmal tumour have been documented. CASE REPORT: We report a case of a malignant proliferating trichilemmal cyst of the scalp in a 57-year-old woman. On the vertex she had a voluminous vegetated and multinodular tumour measuring 7 x 5 cm with spontaneous and abundant bleeding, and another lesion of the scalp corresponding to a trichilemmal cyst. Based on histopathological findings, the case was diagnosed as malignant proliferating trichilemmal cyst. CONCLUSION: Diagnostic, clinicoprognostic and histological features of this tumour are discussed. Treatment is not yet standardized given its rarity.

[Pediatric mandibular ameloblastic fibro-odontoma]. / Fibrome odonto-améloblastique de l'enfant.

INTRODUCTION: Ameloblastic fibro-odontoma (AFO) is a very rare mixed odontogenic tumor (2% of all odontogenic tumors). It is considered as a non-extensive non-aggressive tumor but recurrence and malignant transformation remain possible. We report a voluminous AFO in a child. CASE REPORT: A 7-year-old boy presented with a right mandibular and facial swelling. The panoramic radiograph and CT scan revealed a voluminous unilocular radiolucent lesion (10 cm) with an impacted tooth that involved the ramus area and the angle of the right mandible. Enucleation and curettage was performed with a good outcome. There was no recurrence at ten months. DISCUSSION: This case is exceptional because of the young patient's age, the large tumor diameter, and the good outcome after ten months. This tumor occurs more often in the posterior region of the mandible and is frequently seen in the first two decades of life. The diagnosis is made on radio-clinical data and may be confirmed by histology. A surgical treatment with complete tumor removal is recommended to prevent recurrence.

Rectal leiomyosarcoma: report on two cases and a practical approach to differential diagnosis.

Rectal leiomyosarcoma is an uncommon malignancy. Herein, we describe the clinicopathological features and biological behaviour of these tumours, and provide a practical approach to differential diagnosis, particularly with gastrointestinal stromal tumours (GIST). We report two cases in elderly men. In the first, the lesion was 2 cm from the anal sphincter, while it was located in the rectal ampulla in the second case. Histologically, both tumours were characterized by pleiomorphic, large spindle cells, presenting numerous mitoses and marked nuclear atypia. Immunohistochemical analysis showed that tumour cells coexpressed both actin and desmin, whereas CD117 and S100 protein were negative. The final diagnosis was leiomyosarcoma. One of the patients died of pulmonary metastasis within six months. The second patient had bone metastasis, but was lost to follow up. This report underlines the potential diagnostic problems raised by rectal leiomyosarcoma and emphasizes the role of immunohistochemistry in achieving correct diagnosis, which has important clinical, therapeutic and prognostic consequences.

[Cecal perforation in a kidney transplant patient: disseminated histoplasmosis]. / Perforation caecale chez un transplanté rénal.

Disseminated histoplasmosis is a fungal infection caused by Histoplasma capsulatum. It often involves immunodeficient patients and can occur in two forms, i.e., the large- and small-celled variants. The purpose of this report is to describe a case of disseminated histoplasmosis with cutaneous and digestive involvement observed four years after kidney transplantation in a man from Senegal. The patient developed severe sepsis secondary to colonic perforation. Outcome was fatal due to delayed diagnosis and extent of disease.

No evidence of the APC D1822V missense variant's pathogenicity in Tunisian patients with sporadic colorectal cancer.

OBJECTIVES: Sporadic colorectal cancer is influenced by numerous single nucleotide polymorphisms (SNPs), each with minor effects on the cancer risk. This study seeks to determine whether there is any association of the I1307K, E1317Q and D1822V variants within the Adenomatous polyposis coli gene (APC) and risk to develop colorectal cancer in Tunisian population. METHODS: Direct sequencing was used to investigate three SNPs in the APC in 48 Tunisian sporadic colorectal cancer cases and 63 controls. RESULTS: There was no statistically significant association between the I1307K, E1317Q and D1822V variants investigated and colorectal cancer risk. CONCLUSION: The lack of association may show that these variants selected for this study are not involved in the colorectal carcinogenic process. Otherwise, the eventual biological effect is so little to go undetected, unless increasing the sample size.

Epidemiological study, immunohistochemistry and in situ hybridization studies of nasopharyngeal carcinomas: a Tunisian report.

Nasopharyngeal carcinomas (NPC) are a significant problem of public health in Tunisia. They are particular because of their characteristic geographic distribution. The aims of this study were, first, to appreciate the presence of Epstein-Barr virus (EBV) genome by immunohistochemistry (IHC) and in situ hybridization (ISH) and to compare their benefits to NPC diagnosis and, secondly, to verify the relation between NPC and factors bound to the food and environment conditions. Biopsies, recruited at the department of pathology of EPS Charles Nicolle at Tunis, were analyzed for EBV genome presence by ISH of EBV-encoded small RNA1 (EBER1). IHC was done with encoded nuclear antigen (EBNA1), latent membrane proteins (LMP1), and antigen BZ1 anti-Z EBV-replication activator (ZEBRA). An epidemiological study based upon the analysis of a detailed questionnaire submitted to patients (all from the north of Tunisia) and 60 witnesses was done. The statistic analysis was realised by SPSS Windows 11.5 Advanced Statistics. All samples were classified as Undifferentiated Carcinoma of Nasopharyngeal type (UCNT). We found a sex ratio of 2 with a bimodal repartition. ISH showed 96.6% positive samples. IHC revealed the EBV in 90% of cases and 66.7%, respectively, with EBNA1 and LMP1. The statistic analysis showed a meaningful relation (P<0.05, OR>3) between NPC and dietary factors (spices and piquant condiment), alcohol and the water quality.

Urothelial carcinoma with plasmocytoid component.

Plasmocytoid urothelial carcinoma is a rare subtype of tumour of the urinary bladder. Its clinical and histopathological features have not been well characterized. There are few reports of this type of tumour. We report a case of 65-year-old man who was operated in our department for bladder tumour. The pathological diagnosis was high-grade urothelial carcinoma with plasmocytoid component. The patient died shortly thereafter from liver and bone metastasis.

[Prognostic value of morphologic subdivision of papillary renal cell carcinoma and MUC1 expression]. / Intérêt pronostique du sous-typage morphologique des tumeurs tubulopapillaires du rein et expression de MUC1.

OBJECTIVE: The purpose of our study was to demonstrate the prognostic value of morphologic subdivision of papillary renal cell carcinoma and compare MUC1 expression in two types. MATERIALS AND METHODS: The present retrospective study included 30 cases of papillary renal cell carcinoma based on review of histology slides. The histologic type, Führman grade and stage pTNM were specified. Immunohistochemistry was performed in 22 cases using antibody MUC1. RESULTS: Patients were 23 men and seven women with a mean age of 59.6 years. Eleven tumors were type 1 and 19 were type 2. Type 2 tumors were significantly associated with a higher Führman grade (p = 0.0002). We showed a differential expression of MUC1, which is frequently expressed in the type 1. A local recurrence occurred in one case, lung metastasis in the second one and both tumors were type 2. The survival rate without recurrence and metastasis was 100 % in the type 1. It was 79 % at 12 months and 59 % at 24 months in the type 2. MUC1 expression was correlated with the outcome. CONCLUSION: Type 2 tumors are associated with a higher Führman grade than type 1 and MUC1 expression is more frequent in type 1 and correlated with outcome.

[Immunohistochemical study of endometrial stromal sarcoma and smooth-muscle tumors of the uterus]. / Etude immunohistochimique des sarcomes du stroma endométrial et des tumeurs musculaires lisses de l'utérus.

OBJECTIVES: Positive and differential diagnoses of mesenchymal tumors of the uterus may be sometimes problematic. The purpose of this study was to evaluate the utility of a panel of antibodies in this diagnosis. MATERIALS AND METHODS: The expression of AML, desmin, h-caldesmon and CD10 was studied in nine endometrial stromal sarcomas (SSE), two leiomyosarcomas (LMS) and 10 highly-cellular leiomyoma (HCL). RESULTS: AML positivity was found in five SSE, in all HCL and in only one LMS. Desmin expression was found in two SSE, in all HCL and LMS. H-caldesmon was negative in all SSE, positive in all HCL and in one case of LMS. CD10 was expressed in two-third of SSE. However, neither HCL nor LMS was marqued. CONCLUSION: CD10 is the most specific antibody of SSE. It seems to have the best value in the diagnosis of mesenchymal tumors in association with desmin and h-caldesmon, specific markers of smooth-muscle differentiation.

[Gastric cryptosporidiosis revealing a small cell lung carcinoma (Tunisia)]. / Cryptosporidiose gastrique révélant un carcinome a petites cellules bronchique (Tunisie).

Cryptosporidium, agent of cryptosporidiosis, is an ubiquitous protozoan organism causing diarrhoea especially in severe immunosuppressed patients. Cryptosporidium has been detected with increasing frequency in the gastrointestinal tract, but involvement of the stomach is rarely reported and discloses an underlying immunodeficiency state. We report the case of 67-year-old man, a heavy smoker, who presented with a history of epigastric pain with an altered general condition. Upper gastrointestinal endoscopy showed no significant mucosal abnormalities. The biopsy revealed a chronic active gastritis with Cryptosporidium parasites lining cryptic epithelium. Systematic chest X ray showed a right suspect parenchymatous opacity. Bronchoscopy with multiple biopsies concluded to a small cell lung carcinoma. Through this rare initial manifestation of immunocompromised state related to cancer we will discuss the role of gastrointestinal endoscopy with biopsies in the diagnosis of cryptosporidiosis.

[Proliferating Brenner tumor: case report]. / Tumeur de Brenner proliférante: à propos d'un cas.

Brenner tumors account for only 1 to 2% of ovarian tumors. Proliferating Brenner tumors are characterized by an epithelial proliferation, resembling superficial well-differentiated urothelial carcinomas. A single 29-year-old patient was operated for an ovarian tumor. On gross examination, the tumor, which originated in the left ovary, was cystic multilocular and presented polyoid excrescences. Definitive histological exam showed a papillary proliferation lined by transitional cells without stroma infiltration. Mucinous cells lined some cavities. The diagnosis of proliferating Brenner tumor was made. Clinical and pathological characteristics of this rare entity will be discussed.

Primary umbilical endometriosis: a rare variant of extragenital endometriosis.

Endometriosis is defined as the presence of extra-uterine endometrial tissue. The prevalence rate of umbilical endometriosis ranges from 0.5 to 1.0% of all patients with extragenital endometriosis. In this report, we present a case of primary umbilical endometriosis to highlight the challenges encountered during diagnosis. A nulliparous 39-year-old woman presented to our department with a 2-year history of a tender, painful and non-reducible, firm umbilical mass that enlarged slowly reaching 2 cm in diameter. She had never been pregnant nor had any abdominal surgery. There was no sequential bleeding. Ultrasound of the umbilical region showed a well defined, oval shaped anechoic area. Histological examination on a cutaneous biopsy concluded umbilical endometriosis. The patient was subsequently referred to a gynecologist and underwent surgery with en bloc excision of the lesion. Generally, umbilical endometriosis presents as a roundish tumuor that can either partly or completely occupy the umbilical scar with intermittent bleeding. Characteristically, the mass increases with the menstrual cycle, becoming more evident and usually harder and is associated with cyclic pain. Its pathogenesis remains uncertain. Clinical diagnosis is difficult, and umbilical endometriosis can be easily confused with other conditions such as benign and malignant tumours. Ultrasound examination is useful, and surgical excision is the treatment of choice. Several cases of malignant transformation have also been described.

Granuloma annulare.

AIM: Granuloma annulare (GA) is a palisading granulomatous skin disease. It is a relatively common dermatosis typically characterized by an annular arrangement of erythematous or flesh-coloured papules. The aim of this study was to assess the epidemiological and clinical pattern of GA and its management. METHODS: A retrospective study dealing with 35 cases of GA collected during an 11-year period from 1995 to 2005 was carried out at the Dermatology Department of Charles Nicolle's Hospital of Tunis. RESULTS: There were 24 females and 11 males including 13 children. Four clinical patterns were observed: localized GA with annular shaped lesions in 22 cases (62.8%), subcutaneous GA in 9 cases (25.7%) with 4 children, combination of localized and subcutaneous patterns in one child (2.8%), generalized GA in one adult-patient (2.8%) and combination of generalized and subcutaneous patterns in 2 cases (5.7%). Associated diabetes mellitus was seen in 9 adults (25%) and thyroid diseases in 2 other adults (5.7%). Topical steroids were proposed in 9 cases with partial resolution in 6 of them. Four patients were treated by hydroxychloroquine with an improvement in 3 of them. Two patients (generalized GA with subcutaneous form and subcutaneous GA) were treated by dapsone with partial clearing. CONCLUSION: In these series, GA has the same epidemiological and clinical pattern as other series of the literature. Subcutaneous form had a pseudo-rheumatoid presentation in adults and an exclusive involvement of children when it is localized on the scalp. Associated systemic diseases, especially diabetes mellitus, are frequent in the series here observed.

Low-dose X-ray depilatory treatment induces trichoblastic tumors of the scalp.

BACKGROUND: Depilatory radiotherapy for ringworm was largely used before antifungals were available. Patients who underwent this treatment are at high risk of developing scalp tumors or other cancers. The aim of this study was to characterize scalp tumors occurring after X-ray therapy for ringworm. METHODS: We included cases of postradiotherapy scalp tumors recorded at the Dermatology Department of the Charles Nicolle Hospital, Tunis between 1988 and 2001. We recorded clinical descriptions and all cases were resubmitted to microscopic analysis. RESULTS: Sixty-one tumors occurred in 33 men and 12 women with a mean age of 49.8 years. Radiodermatitis was present in 21% of patients. Tumors were basal cell carcinomas in 47 cases, trichoblastomas in 10 cases and trichoblastic carcinomas in 4 cases. Twelve patients had 2-5 tumors, with combinations of tumor types in 3 of them. Mean delay of onset of tumors after radiotherapy was 39.4 years in basal cell carcinoma cases, 38.3 years in trichoblastoma cases and 35.6 years in trichoblastic carcinoma cases. CONCLUSIONS: This series shows that although basal cell carcinoma is the most frequent tumor in this situation, trichoblastomas are common. We describe, for the first time, radio-induced trichoblastic carcinomas. Trichoblastic tumors have not yet been described in this context because this concept is relatively recent.

Segmental infarction of the testis: an exceptional complication of diabetes microangiopathy.

We report a case of segmental infarction of the testis in a 55-year-old man. Past medical history included 12 years of type II diabetes and hypertension. The patient presented with a 2-month history of testicular pain and was found clinically and sonographically to have a testicular tumour. The pathological examination of the partial orchiectomy specimen revealed segmental infarction of the testicle secondary to diabetes microangiopathy. We propose diabetes microangiopathy as a localization and aetiology of segmental testicular infarction. A possible testicular sparing procedure through an inguinal approach may be considered in cases of testicular masses for which the clinical and imaging findings are suggestive of focal testicular infarction.

[Adenoid cystic carcinoma of the trachea]. / Carcinome adénoïde kystique de la trachée.

UNLABELLED: Cystic adenoid carcinoma of the trachea, also called cylindroma, is a rare malignant tumor accounting for 1% of all respiratory tract cancers. OBJECTIVES: To recall through two observations and a review of the literature, various epidemiologic anatomical clinical aspects, and evolutionary of cystic glandular carcinome of the respiratory tracts. We report 2 cases of cystic glandular carcinoma of the trachea. CLINICAL CASE: They were two women of 60 and 49 year old who came with a deterioration of the general status with dyspnea, dysphonia and/or dysphagia. Radiological explorations visualized, in the first case, a tumoral process of the superior half of the trachea and, in the second case, a tumoral circumferential process budding in the sub-glottic area and reaching the first rings of the trachea. The two patients had a removal of the tumour An auxiliary radiotherapy was indicated in the first case. CONCLUSION: Cystic adenoid carcinomas of the trachea are rare tumours. Their diagnosis is based on the bronchial fibroscopy associated with the biopsy. The optimal treatment is surgical associated with the radiotherapy. The palliative treatments keep a place among inoperable patients.